-
1
-
-
0035862754
-
Expression of expanded repeat androgen receptor produces neurologic disease in transgenic mice
-
Abel A., Walcott J., Woods J., Duda J., and Merry D.E. Expression of expanded repeat androgen receptor produces neurologic disease in transgenic mice. Hum. Mol. Genet 10 (2001) 107-116
-
(2001)
Hum. Mol. Genet
, vol.10
, pp. 107-116
-
-
Abel, A.1
Walcott, J.2
Woods, J.3
Duda, J.4
Merry, D.E.5
-
2
-
-
0037444446
-
Heat shock protein 70 chaperone overexpression ameliorates phenotypes of the spinal and bulbar muscular atrophy transgenic mouse model by reducing nuclear-localized mutant androgen receptor protein
-
Adachi H., Katsuno M., Minamiyama M., Sang C., Pagoulatos G., Angelidis C., Kusakabe M., Yoshiki A., Kobayashi Y., Doyu M., and Sobue G. Heat shock protein 70 chaperone overexpression ameliorates phenotypes of the spinal and bulbar muscular atrophy transgenic mouse model by reducing nuclear-localized mutant androgen receptor protein. J. Neurosci 23 (2003) 2203-2211
-
(2003)
J. Neurosci
, vol.23
, pp. 2203-2211
-
-
Adachi, H.1
Katsuno, M.2
Minamiyama, M.3
Sang, C.4
Pagoulatos, G.5
Angelidis, C.6
Kusakabe, M.7
Yoshiki, A.8
Kobayashi, Y.9
Doyu, M.10
Sobue, G.11
-
3
-
-
0033978812
-
Sensory involvement in spinal-bulbar muscular atrophy (Kennedy's disease)
-
Antonini G., Gragnani F., Romaniello A., Pennisi E.M., Morino S., Ceschin V., Santoro L., and Cruccu G. Sensory involvement in spinal-bulbar muscular atrophy (Kennedy's disease). Muscle Nerve 23 (2000) 252-258
-
(2000)
Muscle Nerve
, vol.23
, pp. 252-258
-
-
Antonini, G.1
Gragnani, F.2
Romaniello, A.3
Pennisi, E.M.4
Morino, S.5
Ceschin, V.6
Santoro, L.7
Cruccu, G.8
-
4
-
-
0036501074
-
Molecular chaperones enhance the degradation of expanded polyglutamine repeat androgen receptor in a cellular model of spinal and bulbar muscular atrophy
-
Bailey C.K., Andriola I.F., Kampinga H.H., and Merry D.E. Molecular chaperones enhance the degradation of expanded polyglutamine repeat androgen receptor in a cellular model of spinal and bulbar muscular atrophy. Hum. Mol. Genet 11 (2002) 515-523
-
(2002)
Hum. Mol. Genet
, vol.11
, pp. 515-523
-
-
Bailey, C.K.1
Andriola, I.F.2
Kampinga, H.H.3
Merry, D.E.4
-
5
-
-
0029802619
-
Androgen receptors in prostate cancer
-
Bentel J.M., and Tilley W.D. Androgen receptors in prostate cancer. J. Endocrinol 151 (1996) 1-11
-
(1996)
J. Endocrinol
, vol.151
, pp. 1-11
-
-
Bentel, J.M.1
Tilley, W.D.2
-
6
-
-
0026894334
-
Moderate instability of the trinucleotide repeat in spino bulbar muscular atrophy
-
Biancalana V., Serville F., Pommier J., Julien J., Hanauer A., and Mandel J.L. Moderate instability of the trinucleotide repeat in spino bulbar muscular atrophy. Hum. Mol. Genet 1 (1992) 255-258
-
(1992)
Hum. Mol. Genet
, vol.1
, pp. 255-258
-
-
Biancalana, V.1
Serville, F.2
Pommier, J.3
Julien, J.4
Hanauer, A.5
Mandel, J.L.6
-
7
-
-
0028878844
-
Stability of an expanded trinucleotide repeat in the androgen receptor gene in transgenic mice
-
Bingham P.M., Scott M.O., Wang S., McPhaul M.J., Wilson E.M., Garbern J.Y., Merry D.E., and Fischbeck K.H. Stability of an expanded trinucleotide repeat in the androgen receptor gene in transgenic mice. Nat. Genet 9 (1995) 191-196
-
(1995)
Nat. Genet
, vol.9
, pp. 191-196
-
-
Bingham, P.M.1
Scott, M.O.2
Wang, S.3
McPhaul, M.J.4
Wilson, E.M.5
Garbern, J.Y.6
Merry, D.E.7
Fischbeck, K.H.8
-
8
-
-
0344507132
-
Up-regulation of protein chaperones preserves viability of cells expressing toxic Cu/Zn-superoxide dismutase mutants associated with amyotrophic lateral sclerosis
-
Bruening W., Roy J., Giasson B., Figlewicz D.A., Mushynski W.E., and Durham H.D. Up-regulation of protein chaperones preserves viability of cells expressing toxic Cu/Zn-superoxide dismutase mutants associated with amyotrophic lateral sclerosis. J. Neurochem 72 (1999) 693-699
-
(1999)
J. Neurochem
, vol.72
, pp. 693-699
-
-
Bruening, W.1
Roy, J.2
Giasson, B.3
Figlewicz, D.A.4
Mushynski, W.E.5
Durham, H.D.6
-
9
-
-
0032544674
-
Aggregation and motor neuron toxicity of an ALS-linked SOD1 mutant independent from wild-type SOD1
-
Bruijn L.I., Houseweart M.K., Kato S., Anderson K.L., Anderson S.D., Ohama E., Reaume A.G., Scott R.W., and Cleveland D.W. Aggregation and motor neuron toxicity of an ALS-linked SOD1 mutant independent from wild-type SOD1. Science 281 (1998) 1851-1854
-
(1998)
Science
, vol.281
, pp. 1851-1854
-
-
Bruijn, L.I.1
Houseweart, M.K.2
Kato, S.3
Anderson, K.L.4
Anderson, S.D.5
Ohama, E.6
Reaume, A.G.7
Scott, R.W.8
Cleveland, D.W.9
-
10
-
-
0031985869
-
Truncated forms of the androgen receptor are associated with polyglutamine expansion in X-linked spinal and bulbar muscular atrophy
-
Butler R., Leigh P.N., McPhaul M.J., and Gallo J.-M. Truncated forms of the androgen receptor are associated with polyglutamine expansion in X-linked spinal and bulbar muscular atrophy. Hum. Mol. Genet 7 (1998) 121-127
-
(1998)
Hum. Mol. Genet
, vol.7
, pp. 121-127
-
-
Butler, R.1
Leigh, P.N.2
McPhaul, M.J.3
Gallo, J.-M.4
-
11
-
-
0034884320
-
Androgen-induced up-regulation of tubulin isoforms in neuroblastoma cells
-
Butler R., Leigh P.N., and Gallo J.-M. Androgen-induced up-regulation of tubulin isoforms in neuroblastoma cells. J. Neurochem 78 (2001) 854-861
-
(2001)
J. Neurochem
, vol.78
, pp. 854-861
-
-
Butler, R.1
Leigh, P.N.2
Gallo, J.-M.3
-
12
-
-
0028033594
-
The length and location of CAG trinucleotide repeats in the androgen receptor N-terminal domain affect transactivation function
-
Chamberlain N.L., Driver E.D., and Miesfeld R.L. The length and location of CAG trinucleotide repeats in the androgen receptor N-terminal domain affect transactivation function. Nucleic Acid Res 22 (1994) 3181-3186
-
(1994)
Nucleic Acid Res
, vol.22
, pp. 3181-3186
-
-
Chamberlain, N.L.1
Driver, E.D.2
Miesfeld, R.L.3
-
13
-
-
0031838352
-
Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1
-
Cummings C.J., Mancini M.A., Antalffy B., DeFranco D.B., Orr H.T., and Zoghbi H.Y. Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1. Nat. Genet 19 (1998) 148-154
-
(1998)
Nat. Genet
, vol.19
, pp. 148-154
-
-
Cummings, C.J.1
Mancini, M.A.2
Antalffy, B.3
DeFranco, D.B.4
Orr, H.T.5
Zoghbi, H.Y.6
-
14
-
-
0035394668
-
Over-expression of inducible HSP70 chaperone suppresses neuropathology and improves motor function in SCA1 mice
-
Cummings C.J., Sun Y., Opal P., Antalffy B., Mestril R., Orr H.T., Dillmann W.H., and Zoghbi H.Y. Over-expression of inducible HSP70 chaperone suppresses neuropathology and improves motor function in SCA1 mice. Hum. Mol. Genet 10 (2001) 1511-1518
-
(2001)
Hum. Mol. Genet
, vol.10
, pp. 1511-1518
-
-
Cummings, C.J.1
Sun, Y.2
Opal, P.3
Antalffy, B.4
Mestril, R.5
Orr, H.T.6
Dillmann, W.H.7
Zoghbi, H.Y.8
-
15
-
-
0028089903
-
Decrease in androgen binding and effect of androgen treatment in a case of X-linked bulbospinal neuronopathy
-
Danek A., Witt T.N., Mann K., Schweikert H.U., Romalo G., La Spada A.R., and Fischbeck K.H. Decrease in androgen binding and effect of androgen treatment in a case of X-linked bulbospinal neuronopathy. Clin. Investig 72 (1994) 892-897
-
(1994)
Clin. Investig
, vol.72
, pp. 892-897
-
-
Danek, A.1
Witt, T.N.2
Mann, K.3
Schweikert, H.U.4
Romalo, G.5
La Spada, A.R.6
Fischbeck, K.H.7
-
16
-
-
0037113310
-
Ligand-dependent aggregation of polyglutamine-expanded androgen receptor in neuronal cells
-
Darrington R.S., Butler R., Leigh P.N., McPhaul M.J., and Gallo J.-M. Ligand-dependent aggregation of polyglutamine-expanded androgen receptor in neuronal cells. Neuroreport 13 (2002) 2117-2120
-
(2002)
Neuroreport
, vol.13
, pp. 2117-2120
-
-
Darrington, R.S.1
Butler, R.2
Leigh, P.N.3
McPhaul, M.J.4
Gallo, J.-M.5
-
17
-
-
18544410106
-
Formation of neuronal inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation
-
Davies S.W., Turmaine M., Cozens B.A., DiFiglia M., Sharp A.H., Ross C.A., Scherzinger E., Wanker E.E., Mangiarini L., and Bates G.P. Formation of neuronal inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation. Cell 90 (1997) 537-548
-
(1997)
Cell
, vol.90
, pp. 537-548
-
-
Davies, S.W.1
Turmaine, M.2
Cozens, B.A.3
DiFiglia, M.4
Sharp, A.H.5
Ross, C.A.6
Scherzinger, E.7
Wanker, E.E.8
Mangiarini, L.9
Bates, G.P.10
-
18
-
-
18544389189
-
A comprehensive endocrine description of Kennedy's disease revealing androgen insensitivity linked to CAG repeat length
-
Dejager S., Bry-Gauillard H., Bruckert E., Eymard B., Salachas F., LeGuern E., Tardieu S., Chadarevian R., Giral P., and Turpin G. A comprehensive endocrine description of Kennedy's disease revealing androgen insensitivity linked to CAG repeat length. J. Clin. Endocrinol. Metab 87 (2002) 3893-3901
-
(2002)
J. Clin. Endocrinol. Metab
, vol.87
, pp. 3893-3901
-
-
Dejager, S.1
Bry-Gauillard, H.2
Bruckert, E.3
Eymard, B.4
Salachas, F.5
LeGuern, E.6
Tardieu, S.7
Chadarevian, R.8
Giral, P.9
Turpin, G.10
-
19
-
-
0030752709
-
Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain
-
DiFiglia M., Sapp E., Chase K.O., Davies S.W., Bates G.P., Vonsattel J.P., and Aronin N. Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain. Science 277 (1997) 1990-1993
-
(1997)
Science
, vol.277
, pp. 1990-1993
-
-
DiFiglia, M.1
Sapp, E.2
Chase, K.O.3
Davies, S.W.4
Bates, G.P.5
Vonsattel, J.P.6
Aronin, N.7
-
20
-
-
0026456689
-
Severity of X-linked recessive bulbospinal neuronopathy correlates with size of the tandem CAG repeat in androgen receptor gene
-
Doyu M., Sobue G., Mukai E., Kachi T., Yasuda T., Mitsuma T., and Takahashi A. Severity of X-linked recessive bulbospinal neuronopathy correlates with size of the tandem CAG repeat in androgen receptor gene. Ann. Neurol 32 (1992) 707-710
-
(1992)
Ann. Neurol
, vol.32
, pp. 707-710
-
-
Doyu, M.1
Sobue, G.2
Mukai, E.3
Kachi, T.4
Yasuda, T.5
Mitsuma, T.6
Takahashi, A.7
-
21
-
-
0034959263
-
Clinical electrophysiology of the upper and lower motor neuron in amyotrophic lateral sclerosis
-
Eisen A. Clinical electrophysiology of the upper and lower motor neuron in amyotrophic lateral sclerosis. Semin. Neurol 21 (2001) 141-145
-
(2001)
Semin. Neurol
, vol.21
, pp. 141-145
-
-
Eisen, A.1
-
22
-
-
0031035995
-
The characteristic electrodiagnostic features of Kennedy's disease
-
Ferrante M.A., and Wilbourn A.J. The characteristic electrodiagnostic features of Kennedy's disease. Muscle Nerve 20 (1997) 323-329
-
(1997)
Muscle Nerve
, vol.20
, pp. 323-329
-
-
Ferrante, M.A.1
Wilbourn, A.J.2
-
23
-
-
0024976878
-
Ubiquitous variations in nerves
-
Gallo J.-M., and Anderton B.H. Ubiquitous variations in nerves. Nature 337 (1989) 687-688
-
(1989)
Nature
, vol.337
, pp. 687-688
-
-
Gallo, J.-M.1
Anderton, B.H.2
-
24
-
-
0030023666
-
Testosterone therapy and the pathogenesis of Kennedy's disease (X-linked bulbospinal muscular atrophy)
-
Goldenberg J.N., and Bradley W.G. Testosterone therapy and the pathogenesis of Kennedy's disease (X-linked bulbospinal muscular atrophy). J. Neurol. Sci 135 (1996) 158-161
-
(1996)
J. Neurol. Sci
, vol.135
, pp. 158-161
-
-
Goldenberg, J.N.1
Bradley, W.G.2
-
25
-
-
0026544312
-
Androgen resistance - the clinical and molecular spectrum
-
Griffin J.E. Androgen resistance - the clinical and molecular spectrum. N. Engl. J. Med 326 (1992) 611-618
-
(1992)
N. Engl. J. Med
, vol.326
, pp. 611-618
-
-
Griffin, J.E.1
-
26
-
-
0037734370
-
Mutations in dynein link motor neuron degeneration to defects in retrograde transport
-
Hafezparast M., Klocke R., Ruhrberg C., Marquardt A., Ahmad-Annuar A., Bowen S., Lalli G., Witherden A.S., Hummerich H., Nicholson S., Morgan P.J., Oozageer R., Priestley J.V., Averill S., King V.R., Ball S., Peters J., Toda T., Yamamoto A., Hiraoka Y., Augustin M., Korthaus D., Wattler S., Wabnitz P., Dickneite C., Lampel S., Boehme F., Peraus G., Popp A., Rudelius M., Schlegel J., Fuchs H., de Angelis M.H., Schiavo G., Shima D.T., Russ A.P., Stumm G., Martin J.E., and Fisher E.M. Mutations in dynein link motor neuron degeneration to defects in retrograde transport. Science 300 (2003) 808-812
-
(2003)
Science
, vol.300
, pp. 808-812
-
-
Hafezparast, M.1
Klocke, R.2
Ruhrberg, C.3
Marquardt, A.4
Ahmad-Annuar, A.5
Bowen, S.6
Lalli, G.7
Witherden, A.S.8
Hummerich, H.9
Nicholson, S.10
Morgan, P.J.11
Oozageer, R.12
Priestley, J.V.13
Averill, S.14
King, V.R.15
Ball, S.16
Peters, J.17
Toda, T.18
Yamamoto, A.19
Hiraoka, Y.20
Augustin, M.21
Korthaus, D.22
Wattler, S.23
Wabnitz, P.24
Dickneite, C.25
Lampel, S.26
Boehme, F.27
Peraus, G.28
Popp, A.29
Rudelius, M.30
Schlegel, J.31
Fuchs, H.32
de Angelis, M.H.33
Schiavo, G.34
Shima, D.T.35
Russ, A.P.36
Stumm, G.37
Martin, J.E.38
Fisher, E.M.39
more..
-
27
-
-
0020457362
-
X-linked recessive bulbospinal neuronopathy: a report of ten cases
-
Harding A.E., Thomas P.K., Baraitser M., Bradbury P.G., Morgan-Hughes J.A., and Pongsford J.R. X-linked recessive bulbospinal neuronopathy: a report of ten cases. J. Neurol. Neurosurg. Psychiatry 45 (1982) 1012-1019
-
(1982)
J. Neurol. Neurosurg. Psychiatry
, vol.45
, pp. 1012-1019
-
-
Harding, A.E.1
Thomas, P.K.2
Baraitser, M.3
Bradbury, P.G.4
Morgan-Hughes, J.A.5
Pongsford, J.R.6
-
28
-
-
0036219918
-
Androgen receptor (AR) coregulators: an overview
-
Heinlein C.A., and Chang C. Androgen receptor (AR) coregulators: an overview. Endocrinol. Rev 23 (2002) 175-200
-
(2002)
Endocrinol. Rev
, vol.23
, pp. 175-200
-
-
Heinlein, C.A.1
Chang, C.2
-
29
-
-
0030457014
-
Ubiquitin-dependent protein degradation
-
Hochstrasser M. Ubiquitin-dependent protein degradation. Annu. Rev. Genet 30 (1996) 405-439
-
(1996)
Annu. Rev. Genet
, vol.30
, pp. 405-439
-
-
Hochstrasser, M.1
-
30
-
-
0037452775
-
Suberoylanilide hydroxamic acid, a histone deacetylase inhibitor, ameliorates motor deficits in a mouse model of Huntington's disease
-
Hockly E., Richon V.M., Woodman B., Smith D.L., Zhou X., Rosa E., Sathasivam K., Ghazi-Noori S., Mahal A., Lowden P.A., Steffan J.S., Marsh J.L., Thompson L.M., Lewis C.M., Marks P.A., and Bates G.P. Suberoylanilide hydroxamic acid, a histone deacetylase inhibitor, ameliorates motor deficits in a mouse model of Huntington's disease. Proc. Natl Acad. Sci. USA 100 (2003) 2041-2046
-
(2003)
Proc. Natl Acad. Sci. USA
, vol.100
, pp. 2041-2046
-
-
Hockly, E.1
Richon, V.M.2
Woodman, B.3
Smith, D.L.4
Zhou, X.5
Rosa, E.6
Sathasivam, K.7
Ghazi-Noori, S.8
Mahal, A.9
Lowden, P.A.10
Steffan, J.S.11
Marsh, J.L.12
Thompson, L.M.13
Lewis, C.M.14
Marks, P.A.15
Bates, G.P.16
-
31
-
-
0033646625
-
Complex fasciculations and their origin in amyotrophic lateral sclerosis and Kennedy's disease
-
Hirota N., Eisen A., and Weber M. Complex fasciculations and their origin in amyotrophic lateral sclerosis and Kennedy's disease. Muscle Nerve 23 (2000) 1872-1875
-
(2000)
Muscle Nerve
, vol.23
, pp. 1872-1875
-
-
Hirota, N.1
Eisen, A.2
Weber, M.3
-
32
-
-
0033575213
-
The linkage of Kennedy's neuron disease to ARA24, the first identified androgen receptor polyglutamine region-associated coactivator
-
Hsiao P.W., Lin D.L., Nakao R., and Chang C. The linkage of Kennedy's neuron disease to ARA24, the first identified androgen receptor polyglutamine region-associated coactivator. J. Biol. Chem 274 (1999) 20229-20234
-
(1999)
J. Biol. Chem
, vol.274
, pp. 20229-20234
-
-
Hsiao, P.W.1
Lin, D.L.2
Nakao, R.3
Chang, C.4
-
33
-
-
0031774524
-
X-linked recessive bulbospinal neuronopathy: clinical and molecular studies in a Taiwanese family
-
Huang C.C., Chen R.S., Chu N.S., Cheng C.C., Pang C.Y., and Wei Y.H. X-linked recessive bulbospinal neuronopathy: clinical and molecular studies in a Taiwanese family. J. Formos. Med. Assoc 97 (1998) 354-359
-
(1998)
J. Formos. Med. Assoc
, vol.97
, pp. 354-359
-
-
Huang, C.C.1
Chen, R.S.2
Chu, N.S.3
Cheng, C.C.4
Pang, C.Y.5
Wei, Y.H.6
-
34
-
-
0026621091
-
Strong correlation between the number of CAG repeats in androgen receptor genes and the clinical onset of features of spinal and bulbar muscular atrophy
-
Igarashi S., Tanno Y., Onodera O., Yamazaki M., Sato S., Ishikawa A., Miyatani N., Nagashima M., Ishikawa Y., Sahashi K., Ibi T., Miyatake T., and Tsuji S. Strong correlation between the number of CAG repeats in androgen receptor genes and the clinical onset of features of spinal and bulbar muscular atrophy. Neurology 42 (1992) 2300-2302
-
(1992)
Neurology
, vol.42
, pp. 2300-2302
-
-
Igarashi, S.1
Tanno, Y.2
Onodera, O.3
Yamazaki, M.4
Sato, S.5
Ishikawa, A.6
Miyatani, N.7
Nagashima, M.8
Ishikawa, Y.9
Sahashi, K.10
Ibi, T.11
Miyatake, T.12
Tsuji, S.13
-
35
-
-
0034793311
-
Clinical features and skewed X-chromosome inactivation in female carriers of X-linked recessive spinal and bulbar muscular atrophy
-
Ishihara H., Kanda F., Nishio H., Sumino K., and Chihara K. Clinical features and skewed X-chromosome inactivation in female carriers of X-linked recessive spinal and bulbar muscular atrophy. J. Neurol 248 (2001) 856-860
-
(2001)
J. Neurol
, vol.248
, pp. 856-860
-
-
Ishihara, H.1
Kanda, F.2
Nishio, H.3
Sumino, K.4
Chihara, K.5
-
36
-
-
0028557388
-
Androgenic enhancement of motor neuron regeneration
-
Jones K.J. Androgenic enhancement of motor neuron regeneration. Ann. NY Acad. Sci 743 (1994) 141-161
-
(1994)
Ann. NY Acad. Sci
, vol.743
, pp. 141-161
-
-
Jones, K.J.1
-
37
-
-
0028241902
-
Androgenic regulation of tubulin gene expression in axotomized hamster facial motoneurons
-
Jones K.J., and Oblinger M.M. Androgenic regulation of tubulin gene expression in axotomized hamster facial motoneurons. J. Neurosci 14 (1994) 3620-3627
-
(1994)
J. Neurosci
, vol.14
, pp. 3620-3627
-
-
Jones, K.J.1
Oblinger, M.M.2
-
39
-
-
0030797166
-
Pathological characterization of astrocytic hyaline inclusions in familial amyotrophic lateral sclerosis
-
Kato S., Hayashi H., Nakashima K., Nanba E., Kato M., Hirano A., Nakano I., Asayama K., and Ohama E. Pathological characterization of astrocytic hyaline inclusions in familial amyotrophic lateral sclerosis. Am. J. Pathol 151 (1997) 611-620
-
(1997)
Am. J. Pathol
, vol.151
, pp. 611-620
-
-
Kato, S.1
Hayashi, H.2
Nakashima, K.3
Nanba, E.4
Kato, M.5
Hirano, A.6
Nakano, I.7
Asayama, K.8
Ohama, E.9
-
40
-
-
18644379256
-
Testosterone reduction prevents phenotypic expression in a transgenic mouse model of spinal and bulbar muscular atrophy
-
Katsuno M., Adachi H., Kume A., Li M., Nakagomi Y., Niwa H., Sang C., Kobayashi Y., Doyu M., and Sobue G. Testosterone reduction prevents phenotypic expression in a transgenic mouse model of spinal and bulbar muscular atrophy. Neuron 35 (2002) 843-854
-
(2002)
Neuron
, vol.35
, pp. 843-854
-
-
Katsuno, M.1
Adachi, H.2
Kume, A.3
Li, M.4
Nakagomi, Y.5
Niwa, H.6
Sang, C.7
Kobayashi, Y.8
Doyu, M.9
Sobue, G.10
-
41
-
-
0038714285
-
Leuprorelin rescues polyglutamine-dependent phenotypes in a transgenic mouse model of spinal and bulbar muscular atrophy
-
Katsuno M., Adachi H., Doyu M., Minamiyama M., Sang C., Kobayashi Y., Inukai A., and Sobue G. Leuprorelin rescues polyglutamine-dependent phenotypes in a transgenic mouse model of spinal and bulbar muscular atrophy. Nat. Med 9 (2003) 768-773
-
(2003)
Nat. Med
, vol.9
, pp. 768-773
-
-
Katsuno, M.1
Adachi, H.2
Doyu, M.3
Minamiyama, M.4
Sang, C.5
Kobayashi, Y.6
Inukai, A.7
Sobue, G.8
-
43
-
-
0014310582
-
Progressive proximal spinal and bulbar muscular atrophy of late onset: a sex-linked recessive trait
-
Kennedy W.R., Alter M., and Sung J.H. Progressive proximal spinal and bulbar muscular atrophy of late onset: a sex-linked recessive trait. Neurology 18 (1968) 671-680
-
(1968)
Neurology
, vol.18
, pp. 671-680
-
-
Kennedy, W.R.1
Alter, M.2
Sung, J.H.3
-
44
-
-
0032475941
-
Ataxin-1 nuclear localization and aggregation: role in polyglutamine-induced disease in SCA1 transgenic mice
-
Klement I.A., Skinner P.J., Kaytor M.D., Yi H., Hersch S.M., Clark H.B., Zoghbi H.Y., and Orr H.T. Ataxin-1 nuclear localization and aggregation: role in polyglutamine-induced disease in SCA1 transgenic mice. Cell 95 (1998) 41-53
-
(1998)
Cell
, vol.95
, pp. 41-53
-
-
Klement, I.A.1
Skinner, P.J.2
Kaytor, M.D.3
Yi, H.4
Hersch, S.M.5
Clark, H.B.6
Zoghbi, H.Y.7
Orr, H.T.8
-
45
-
-
0024328136
-
Testosterone-induced acceleration of recovery from facial paralysis following crush axotomy of the facial nerve in male hamsters
-
Kujawa K.A., Kinderman N.B., and Jones K.J. Testosterone-induced acceleration of recovery from facial paralysis following crush axotomy of the facial nerve in male hamsters. Exp. Neurol 105 (1989) 80-85
-
(1989)
Exp. Neurol
, vol.105
, pp. 80-85
-
-
Kujawa, K.A.1
Kinderman, N.B.2
Jones, K.J.3
-
46
-
-
0026322290
-
Testosterone differentially regulates the regenerative properties of injured hamster facial motoneurons
-
Kujawa K.A., Emeric E., and Jones K.J. Testosterone differentially regulates the regenerative properties of injured hamster facial motoneurons. J. Neurosci 11 (1991) 3898-3906
-
(1991)
J. Neurosci
, vol.11
, pp. 3898-3906
-
-
Kujawa, K.A.1
Emeric, E.2
Jones, K.J.3
-
47
-
-
0025800526
-
Androgen receptor gene mutations in X-linked spinal and bulbar muscular atrophy
-
La Spada A.R., Wilson E.M., Lubahn D.B., Harding A.E., and Fischbeck K.H. Androgen receptor gene mutations in X-linked spinal and bulbar muscular atrophy. Nature 352 (1991) 77-79
-
(1991)
Nature
, vol.352
, pp. 77-79
-
-
La Spada, A.R.1
Wilson, E.M.2
Lubahn, D.B.3
Harding, A.E.4
Fischbeck, K.H.5
-
48
-
-
0027023516
-
Meiotic stability and genotype-phenotype correlation of the trinucleotide repeat in X-linked spinal and bulbar muscular atrophy
-
La Spada A.R., Roling D.B., Harding A.E., Warner C.L., Spiegel R., Hausmanowa-Petrusewicz I., Yee W.C., and Fischbeck K.H. Meiotic stability and genotype-phenotype correlation of the trinucleotide repeat in X-linked spinal and bulbar muscular atrophy. Nat. Genet 2 (1992) 301-304
-
(1992)
Nat. Genet
, vol.2
, pp. 301-304
-
-
La Spada, A.R.1
Roling, D.B.2
Harding, A.E.3
Warner, C.L.4
Spiegel, R.5
Hausmanowa-Petrusewicz, I.6
Yee, W.C.7
Fischbeck, K.H.8
-
49
-
-
7144256250
-
Androgen receptor YAC transgenic mice carrying CAG 45 alleles show trinucleotide repeat instability
-
La Spada A.R., Peterson K.R., Meadows S.A., McClain M.E., Jeng G., Chmelar R.S., Haugen H.A., Chen K., Singer M.J., Moore D., Trask B.J., Fischbeck K.H., Clegg C.H., and McKnight G.S. Androgen receptor YAC transgenic mice carrying CAG 45 alleles show trinucleotide repeat instability. Hum. Mol. Genet 7 (1998) 959-967
-
(1998)
Hum. Mol. Genet
, vol.7
, pp. 959-967
-
-
La Spada, A.R.1
Peterson, K.R.2
Meadows, S.A.3
McClain, M.E.4
Jeng, G.5
Chmelar, R.S.6
Haugen, H.A.7
Chen, K.8
Singer, M.J.9
Moore, D.10
Trask, B.J.11
Fischbeck, K.H.12
Clegg, C.H.13
McKnight, G.S.14
-
50
-
-
17944370599
-
Polyglutamine-expanded ataxin-7 antagonizes CRX function and induces cone-rod dystrophy in a mouse model of SCA7
-
La Spada A.R., Fu Y.H., Sopher B.L., Libby R.T., Wang X., Li L.Y., Einum D.D., Huang J., Possin D.E., Smith A.C., Martinez R.A., Koszdin K.L., Treuting P.M., Ware C.B., Hurley J.B., Ptacek L.J., and Chen S. Polyglutamine-expanded ataxin-7 antagonizes CRX function and induces cone-rod dystrophy in a mouse model of SCA7. Neuron 31 (2001) 913-927
-
(2001)
Neuron
, vol.31
, pp. 913-927
-
-
La Spada, A.R.1
Fu, Y.H.2
Sopher, B.L.3
Libby, R.T.4
Wang, X.5
Li, L.Y.6
Einum, D.D.7
Huang, J.8
Possin, D.E.9
Smith, A.C.10
Martinez, R.A.11
Koszdin, K.L.12
Treuting, P.M.13
Ware, C.B.14
Hurley, J.B.15
Ptacek, L.J.16
Chen, S.17
-
51
-
-
0037198698
-
Disruption of dynein/dynactin inhibits axonal transport in motor neurons causing late-onset progressive degeneration
-
LaMonte B.H., Wallace K.E., Holloway B.A., Shelly S.S., Ascano J., Tokito M., Van Winkle T., Howland D.S., and Holzbaur E.L. Disruption of dynein/dynactin inhibits axonal transport in motor neurons causing late-onset progressive degeneration. Neuron 34 (2002) 715-727
-
(2002)
Neuron
, vol.34
, pp. 715-727
-
-
LaMonte, B.H.1
Wallace, K.E.2
Holloway, B.A.3
Shelly, S.S.4
Ascano, J.5
Tokito, M.6
Van Winkle, T.7
Howland, D.S.8
Holzbaur, E.L.9
-
52
-
-
0023823645
-
Ubiquitin deposits in anterior horn cells in motor neurone disease
-
Leigh P.N., Anderton B.H., Dodson A., Gallo J.-M., Swash M., and Power D.M. Ubiquitin deposits in anterior horn cells in motor neurone disease. Neurosci. Lett 93 (1988) 197-203
-
(1988)
Neurosci. Lett
, vol.93
, pp. 197-203
-
-
Leigh, P.N.1
Anderton, B.H.2
Dodson, A.3
Gallo, J.-M.4
Swash, M.5
Power, D.M.6
-
53
-
-
0024565972
-
Cytoskeletal abnormalities in motor neuron disease. An immunocytochemical study
-
Leigh P.N., Dodson A., Swash M., Brion J.P., and Anderton B.H. Cytoskeletal abnormalities in motor neuron disease. An immunocytochemical study. Brain 112 (1989) 521-535
-
(1989)
Brain
, vol.112
, pp. 521-535
-
-
Leigh, P.N.1
Dodson, A.2
Swash, M.3
Brion, J.P.4
Anderton, B.H.5
-
54
-
-
0028817671
-
Primary sensory neurons in X-linked recessive bulbospinal neuropathy: histopathology and androgen receptor gene expression
-
Li M., Sobue G., Doyu M., Mukai E., Hashizume Y., and Mitsuma T. Primary sensory neurons in X-linked recessive bulbospinal neuropathy: histopathology and androgen receptor gene expression. Muscle Nerve 18 (1995) 301-308
-
(1995)
Muscle Nerve
, vol.18
, pp. 301-308
-
-
Li, M.1
Sobue, G.2
Doyu, M.3
Mukai, E.4
Hashizume, Y.5
Mitsuma, T.6
-
55
-
-
15144342225
-
Nuclear inclusions of the androgen receptor protein in spinal and bulbar muscular atrophy
-
Li M., Miwa S., Kobayashi Y., Merry D.E., Yamamoto M., Tanaka F., Doyu M., Hashizume Y., Fischbeck K.H., and Sobue G. Nuclear inclusions of the androgen receptor protein in spinal and bulbar muscular atrophy. Ann. Neurol 44 (1998) 249-254
-
(1998)
Ann. Neurol
, vol.44
, pp. 249-254
-
-
Li, M.1
Miwa, S.2
Kobayashi, Y.3
Merry, D.E.4
Yamamoto, M.5
Tanaka, F.6
Doyu, M.7
Hashizume, Y.8
Fischbeck, K.H.9
Sobue, G.10
-
56
-
-
0031682209
-
Nonneuronal nuclear inclusions of androgen receptor protein in spinal and bulbar muscular atrophy
-
Li M., Nakagomi Y., Kobayashi Y., Merry D.E., Tanaka F., Doyu M., Mitsuma T., Hashizume Y., Fischbeck K.H., and Sobue G. Nonneuronal nuclear inclusions of androgen receptor protein in spinal and bulbar muscular atrophy. Am. J. Pathol 153 (1998) 695-701
-
(1998)
Am. J. Pathol
, vol.153
, pp. 695-701
-
-
Li, M.1
Nakagomi, Y.2
Kobayashi, Y.3
Merry, D.E.4
Tanaka, F.5
Doyu, M.6
Mitsuma, T.7
Hashizume, Y.8
Fischbeck, K.H.9
Sobue, G.10
-
57
-
-
0041385579
-
A genome scan for modifiers of age at onset in Huntington disease: The HD MAPS study
-
Li J.L., Hayden M.R., Almqvist E.W., Brinkman R.R., Durr A., Dode C., Morrison P.J., Suchowersky O., Ross C.A., Margolis R.L., Rosenblatt A., Gomez-Tortosa E., Cabrero D.M., Novelletto A., Frontali M., Nance M., Trent R.J., McCusker E., Jones R., Paulsen J.S., Harrison M., Zanko A., Abramson R.K., Russ A.L., Knowlton B., Djousse L., Mysore J.S., Tariot S., Gusella M.F., Wheeler V.C., Atwood L.D., Cupples L.A., Saint-Hilaire M., Cha J.H., Hersch S.M., Koroshetz W.J., Gusella J.F., MacDonald M.E., and Myers R.H. A genome scan for modifiers of age at onset in Huntington disease: The HD MAPS study. Am. J. Hum. Genet 73 (2003) 682-687
-
(2003)
Am. J. Hum. Genet
, vol.73
, pp. 682-687
-
-
Li, J.L.1
Hayden, M.R.2
Almqvist, E.W.3
Brinkman, R.R.4
Durr, A.5
Dode, C.6
Morrison, P.J.7
Suchowersky, O.8
Ross, C.A.9
Margolis, R.L.10
Rosenblatt, A.11
Gomez-Tortosa, E.12
Cabrero, D.M.13
Novelletto, A.14
Frontali, M.15
Nance, M.16
Trent, R.J.17
McCusker, E.18
Jones, R.19
Paulsen, J.S.20
Harrison, M.21
Zanko, A.22
Abramson, R.K.23
Russ, A.L.24
Knowlton, B.25
Djousse, L.26
Mysore, J.S.27
Tariot, S.28
Gusella, M.F.29
Wheeler, V.C.30
Atwood, L.D.31
Cupples, L.A.32
Saint-Hilaire, M.33
Cha, J.H.34
Hersch, S.M.35
Koroshetz, W.J.36
Gusella, J.F.37
MacDonald, M.E.38
Myers, R.H.39
more..
-
58
-
-
0037101834
-
Altered transcriptional regulation in cells expressing the expanded polyglutamine androgen receptor
-
Lieberman A.P., Harmison G., Strand A.D., Olson J.M., and Fischbeck K.H. Altered transcriptional regulation in cells expressing the expanded polyglutamine androgen receptor. Hum. Mol. Genet 11 (2002) 1967-1976
-
(2002)
Hum. Mol. Genet
, vol.11
, pp. 1967-1976
-
-
Lieberman, A.P.1
Harmison, G.2
Strand, A.D.3
Olson, J.M.4
Fischbeck, K.H.5
-
59
-
-
0033995175
-
Polyglutamine expansion down-regulates specific neuronal genes before pathologic changes in SCA1
-
Lin X., Antalffy B., Kang D., Orr H.T., and Zoghbi H.Y. Polyglutamine expansion down-regulates specific neuronal genes before pathologic changes in SCA1. Nat. Neurosci 3 (2000) 157-163
-
(2000)
Nat. Neurosci
, vol.3
, pp. 157-163
-
-
Lin, X.1
Antalffy, B.2
Kang, D.3
Orr, H.T.4
Zoghbi, H.Y.5
-
60
-
-
0028289351
-
Facial action myoclonus in patients with olivopontocerebellar atrophy
-
Lou J.S., Valls-Sole J., Toro C., and Hallett M. Facial action myoclonus in patients with olivopontocerebellar atrophy. Mov. Disord 9 (1994) 223-226
-
(1994)
Mov. Disord
, vol.9
, pp. 223-226
-
-
Lou, J.S.1
Valls-Sole, J.2
Toro, C.3
Hallett, M.4
-
61
-
-
0023738162
-
A filamentous inclusion body within anterior horn neurones in motor neurone disease defined by immunocytochemical localisation of ubiquitin
-
Lowe J., Lennox G., Jefferson D., Morrell K., McQuire D., Gray T., Landon M., Doherty F.J., and Mayer R.J. A filamentous inclusion body within anterior horn neurones in motor neurone disease defined by immunocytochemical localisation of ubiquitin. Neurosci. Lett 94 (1988) 203-210
-
(1988)
Neurosci. Lett
, vol.94
, pp. 203-210
-
-
Lowe, J.1
Lennox, G.2
Jefferson, D.3
Morrell, K.4
McQuire, D.5
Gray, T.6
Landon, M.7
Doherty, F.J.8
Mayer, R.J.9
-
62
-
-
0034702030
-
Decreased expression of striatal signaling genes in a mouse model of Huntington's disease
-
Luthi-Carter R., Strand A., Peters N.L., Solano S.M., Hollingsworth Z.R., Menon A.S., Frey A.S., Spektor B.S., Penney E.B., Schilling G., Ross C.A., Borchelt D.R., Tapscott S.J., Young A.B., Cha J.H., and Olson J.M. Decreased expression of striatal signaling genes in a mouse model of Huntington's disease. Hum. Mol. Genet 9 (2000) 1259-1271
-
(2000)
Hum. Mol. Genet
, vol.9
, pp. 1259-1271
-
-
Luthi-Carter, R.1
Strand, A.2
Peters, N.L.3
Solano, S.M.4
Hollingsworth, Z.R.5
Menon, A.S.6
Frey, A.S.7
Spektor, B.S.8
Penney, E.B.9
Schilling, G.10
Ross, C.A.11
Borchelt, D.R.12
Tapscott, S.J.13
Young, A.B.14
Cha, J.H.15
Olson, J.M.16
-
63
-
-
0028843242
-
Abnormal androgen receptor binding affinity in subjects with Kennedy's disease (spinal and bulbar muscular atrophy)
-
MacLean H.E., Choi W.T., Rekaris G., Warne G.L., and Zajac J.D. Abnormal androgen receptor binding affinity in subjects with Kennedy's disease (spinal and bulbar muscular atrophy). J. Clin. Endocrinol. Metab 80 (1995) 508-516
-
(1995)
J. Clin. Endocrinol. Metab
, vol.80
, pp. 508-516
-
-
MacLean, H.E.1
Choi, W.T.2
Rekaris, G.3
Warne, G.L.4
Zajac, J.D.5
-
65
-
-
0034285017
-
CREB-binding protein sequestration by expanded polyglutamine
-
McCampbell A., Taylor J.P., Taye A.A., Robitschek J., Li M., Walcott J., Merry D., Chai Y., Paulson H., Sobue G., and Fischbeck K.H. CREB-binding protein sequestration by expanded polyglutamine. Hum. Mol. Genet 9 (2000) 2197-2202
-
(2000)
Hum. Mol. Genet
, vol.9
, pp. 2197-2202
-
-
McCampbell, A.1
Taylor, J.P.2
Taye, A.A.3
Robitschek, J.4
Li, M.5
Walcott, J.6
Merry, D.7
Chai, Y.8
Paulson, H.9
Sobue, G.10
Fischbeck, K.H.11
-
66
-
-
0035909999
-
Histone deacetylase inhibitors reduce polyglutamine toxicity
-
McCampbell A., Taye A.A., Whitty L., Penney E., Steffan J.S., and Fischbeck K.H. Histone deacetylase inhibitors reduce polyglutamine toxicity. Proc. Natl Acad. Sci. USA 98 (2001) 15179-15184
-
(2001)
Proc. Natl Acad. Sci. USA
, vol.98
, pp. 15179-15184
-
-
McCampbell, A.1
Taye, A.A.2
Whitty, L.3
Penney, E.4
Steffan, J.S.5
Fischbeck, K.H.6
-
67
-
-
0025720533
-
Androgen resistance caused by mutations in the androgen receptor gene
-
McPhaul M.J., Marcelli M., Tilley W.D., Griffin J.E., and Wilson J.D. Androgen resistance caused by mutations in the androgen receptor gene. FASEB J 5 (1991) 2910-2915
-
(1991)
FASEB J
, vol.5
, pp. 2910-2915
-
-
McPhaul, M.J.1
Marcelli, M.2
Tilley, W.D.3
Griffin, J.E.4
Wilson, J.D.5
-
68
-
-
0027161059
-
Up-regulation of androgen receptor immunoreactivity in the rat brain by androgenic-anabolic steroids
-
Menard C.S., and Harlan R.E. Up-regulation of androgen receptor immunoreactivity in the rat brain by androgenic-anabolic steroids. Brain Res 622 (1993) 226-236
-
(1993)
Brain Res
, vol.622
, pp. 226-236
-
-
Menard, C.S.1
Harlan, R.E.2
-
69
-
-
0037308313
-
Fatigue and abnormal neuromuscular transmission in Kennedy's disease
-
Meriggioli M.N., and Rowin J. Fatigue and abnormal neuromuscular transmission in Kennedy's disease. Muscle Nerve 27 (2003) 249-251
-
(2003)
Muscle Nerve
, vol.27
, pp. 249-251
-
-
Meriggioli, M.N.1
Rowin, J.2
-
70
-
-
0027366138
-
Reduced transcriptional regulatory competence of the androgen receptor in X-linked spinal and bulbar muscular atrophy
-
Mhatre A.N., Trifiro M.A., Kaufman M., Kazemi-Esfarjani P., Figlewicz D., Rouleau G., and Pinsky L. Reduced transcriptional regulatory competence of the androgen receptor in X-linked spinal and bulbar muscular atrophy. Nat. Genet 5 (1993) 184-188
-
(1993)
Nat. Genet
, vol.5
, pp. 184-188
-
-
Mhatre, A.N.1
Trifiro, M.A.2
Kaufman, M.3
Kazemi-Esfarjani, P.4
Figlewicz, D.5
Rouleau, G.6
Pinsky, L.7
-
71
-
-
0037093205
-
Androgen receptor CAG repeats and prostate cancer
-
Nelson K.A., and Witte J.S. Androgen receptor CAG repeats and prostate cancer. Am. J. Epidemiol 155 (2002) 883-890
-
(2002)
Am. J. Epidemiol
, vol.155
, pp. 883-890
-
-
Nelson, K.A.1
Witte, J.S.2
-
72
-
-
0035937523
-
Interference by huntingtin and atrophin-1 with CBP-mediated transcription leading to cellular toxicity
-
Nucifora Jr. F.C., Sasaki M., Peters M.F., Huang H., Cooper J.K., Yamada M., Takahashi H., Tsuji S., Troncoso J., Dawson V.L., Dawson T.M., and Ross C.A. Interference by huntingtin and atrophin-1 with CBP-mediated transcription leading to cellular toxicity. Science 291 (2001) 2423-2428
-
(2001)
Science
, vol.291
, pp. 2423-2428
-
-
Nucifora Jr., F.C.1
Sasaki, M.2
Peters, M.F.3
Huang, H.4
Cooper, J.K.5
Yamada, M.6
Takahashi, H.7
Tsuji, S.8
Troncoso, J.9
Dawson, V.L.10
Dawson, T.M.11
Ross, C.A.12
-
73
-
-
18444403420
-
Interaction between mutant ataxin-1 and PQBP-1 affects transcription and cell death
-
Okazawa H., Rich T., Chang A., Lin X., Waragai M., Kajikawa M., Enokido Y., Komuro A., Kato S., Shibata M., Hatanaka H., Mouradian M.M., Sudol M., and Kanazawa I. Interaction between mutant ataxin-1 and PQBP-1 affects transcription and cell death. Neuron 34 (2002) 701-713
-
(2002)
Neuron
, vol.34
, pp. 701-713
-
-
Okazawa, H.1
Rich, T.2
Chang, A.3
Lin, X.4
Waragai, M.5
Kajikawa, M.6
Enokido, Y.7
Komuro, A.8
Kato, S.9
Shibata, M.10
Hatanaka, H.11
Mouradian, M.M.12
Sudol, M.13
Kanazawa, I.14
-
74
-
-
0025735846
-
Clinical and electrodiagnostic features of X-linked recessive bulbospinal neuronopathy
-
Olney R.K., Aminoff M.J., and So Y.T. Clinical and electrodiagnostic features of X-linked recessive bulbospinal neuronopathy. Neurology 41 (1991) 823-828
-
(1991)
Neurology
, vol.41
, pp. 823-828
-
-
Olney, R.K.1
Aminoff, M.J.2
So, Y.T.3
-
75
-
-
0028283985
-
Glutamine repeats as polar zippers: their possible role in inherited neurodegenerative diseases
-
Perutz M.F., Johnson T., Suzuki M., and Finch J.T. Glutamine repeats as polar zippers: their possible role in inherited neurodegenerative diseases. Proc. Natl Acad. Sci. USA 91 (1994) 5355-5358
-
(1994)
Proc. Natl Acad. Sci. USA
, vol.91
, pp. 5355-5358
-
-
Perutz, M.F.1
Johnson, T.2
Suzuki, M.3
Finch, J.T.4
-
76
-
-
0029898097
-
Sensory involvement in X-linked spino-bulbar muscular atrophy (Kennedy's syndrome): an electrophysiological study
-
Polo A., Teatini F., D'Anna S., Manganotti P., Salviati A., Dallapiccola B., Zanette G., and Rizzuto N. Sensory involvement in X-linked spino-bulbar muscular atrophy (Kennedy's syndrome): an electrophysiological study. J. Neurol 243 (1996) 388-392
-
(1996)
J. Neurol
, vol.243
, pp. 388-392
-
-
Polo, A.1
Teatini, F.2
D'Anna, S.3
Manganotti, P.4
Salviati, A.5
Dallapiccola, B.6
Zanette, G.7
Rizzuto, N.8
-
77
-
-
0042922996
-
Androgen 5-α-reductase type 2 is highly expressed and active in rat spinal cord motor neurones
-
Pozzi P., Bendotti C., Simeoni S., Piccioni F., Guerini V., Marron T.U., Martini L., and Poletti A. Androgen 5-α-reductase type 2 is highly expressed and active in rat spinal cord motor neurones. J. Neuroendocrinol 15 (2003) 882-887
-
(2003)
J. Neuroendocrinol
, vol.15
, pp. 882-887
-
-
Pozzi, P.1
Bendotti, C.2
Simeoni, S.3
Piccioni, F.4
Guerini, V.5
Marron, T.U.6
Martini, L.7
Poletti, A.8
-
78
-
-
0037382240
-
Mutant dynactin in motor neuron disease
-
Puls I., Jonnakuty C., LaMonte B.H., Holzbaur E.L., Tokito M., Mann E., Floeter M.K., Bidus K., Drayna D., Oh S.J., Brown R.H., Ludlow C.L., and Fischbeck K.H. Mutant dynactin in motor neuron disease. Nat. Genet 33 (2003) 455-456
-
(2003)
Nat. Genet
, vol.33
, pp. 455-456
-
-
Puls, I.1
Jonnakuty, C.2
LaMonte, B.H.3
Holzbaur, E.L.4
Tokito, M.5
Mann, E.6
Floeter, M.K.7
Bidus, K.8
Drayna, D.9
Oh, S.J.10
Brown, R.H.11
Ludlow, C.L.12
Fischbeck, K.H.13
-
79
-
-
0017685107
-
Androgen concentration in motor neurons of cranial nerves and spinal cord
-
Sar M., and Stumpf W.E. Androgen concentration in motor neurons of cranial nerves and spinal cord. Science 197 (1977) 77-79
-
(1977)
Science
, vol.197
, pp. 77-79
-
-
Sar, M.1
Stumpf, W.E.2
-
80
-
-
0032475931
-
Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions
-
Saudou F., Finkbeiner S., Devys D., and Greenberg M.E. Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions. Cell 95 (1998) 55-66
-
(1998)
Cell
, vol.95
, pp. 55-66
-
-
Saudou, F.1
Finkbeiner, S.2
Devys, D.3
Greenberg, M.E.4
-
81
-
-
0037056302
-
Expression of X-linked bulbospinal muscular atrophy (Kennedy disease) in two homozygous women
-
Schmidt B.J., Greenberg C.R., Allingham-Hawkins D.J., and Spriggs E.L. Expression of X-linked bulbospinal muscular atrophy (Kennedy disease) in two homozygous women. Neurology 59 (2002) 770-772
-
(2002)
Neurology
, vol.59
, pp. 770-772
-
-
Schmidt, B.J.1
Greenberg, C.R.2
Allingham-Hawkins, D.J.3
Spriggs, E.L.4
-
82
-
-
0031867908
-
Kennedy's disease: unusual molecular pathological and clinical features
-
Shaw P.J., Thagesen H., Tomkins J., Slade J.Y., Usher P., Jackson A., Curtis A., Bushby K., and Ince P.G. Kennedy's disease: unusual molecular pathological and clinical features. Neurology 51 (1998) 252-255
-
(1998)
Neurology
, vol.51
, pp. 252-255
-
-
Shaw, P.J.1
Thagesen, H.2
Tomkins, J.3
Slade, J.Y.4
Usher, P.5
Jackson, A.6
Curtis, A.7
Bushby, K.8
Ince, P.G.9
-
83
-
-
0029927679
-
Intense superoxide dismutase-1 immunoreactivity in intracytoplasmic hyaline inclusions of familial amyotrophic lateral sclerosis with posterior column involvement
-
Shibata N., Hirano A., Kobayashi M., Siddique T., Deng H.X., Hung W.Y., Kato T., and Asayama K. Intense superoxide dismutase-1 immunoreactivity in intracytoplasmic hyaline inclusions of familial amyotrophic lateral sclerosis with posterior column involvement. J. Neuropathol. Exp. Neurol 55 (1996) 481-490
-
(1996)
J. Neuropathol. Exp. Neurol
, vol.55
, pp. 481-490
-
-
Shibata, N.1
Hirano, A.2
Kobayashi, M.3
Siddique, T.4
Deng, H.X.5
Hung, W.Y.6
Kato, T.7
Asayama, K.8
-
84
-
-
0025232316
-
Distribution of androgen and estrogen receptor mRNA-containing cells in the rat brain: an in situ hybridization study
-
Simerly R.B., Chang C., Muramatsu M., and Swanson L.W. Distribution of androgen and estrogen receptor mRNA-containing cells in the rat brain: an in situ hybridization study. J. Comp. Neurol 294 (1990) 76-95
-
(1990)
J. Comp. Neurol
, vol.294
, pp. 76-95
-
-
Simerly, R.B.1
Chang, C.2
Muramatsu, M.3
Swanson, L.W.4
-
85
-
-
0024586435
-
X-linked recessive bulbospinal neuropathy. A clinicopathological study
-
Sobue G., Hashizume Y., Mukai E., Hirayama M., Mitsuma T., and Takahashi A. X-linked recessive bulbospinal neuropathy. A clinicopathological study. Brain 112 (1989) 209-232
-
(1989)
Brain
, vol.112
, pp. 209-232
-
-
Sobue, G.1
Hashizume, Y.2
Mukai, E.3
Hirayama, M.4
Mitsuma, T.5
Takahashi, A.6
-
86
-
-
0027291710
-
Subclinical phenotypic expressions in heterozygous females of X-linked recessive bulbospinal neuronopathy
-
Sobue G., Doyu M., Kachi T., Yasuda T., Mukai E., Kumagai T., and Mitsuma T. Subclinical phenotypic expressions in heterozygous females of X-linked recessive bulbospinal neuronopathy. J. Neurol. Sci 117 (1993) 74-78
-
(1993)
J. Neurol. Sci
, vol.117
, pp. 74-78
-
-
Sobue, G.1
Doyu, M.2
Kachi, T.3
Yasuda, T.4
Mukai, E.5
Kumagai, T.6
Mitsuma, T.7
-
87
-
-
0036894940
-
X-linked bulbospinal neuronopathy: Kennedy Disease
-
Sperfeld A.D., Karitzky J., Brummer D., Schreiber H., Haussler J., Ludolph A.C., and Hanemann C.O. X-linked bulbospinal neuronopathy: Kennedy Disease. Arch. Neurol 59 (2002) 1921-1926
-
(2002)
Arch. Neurol
, vol.59
, pp. 1921-1926
-
-
Sperfeld, A.D.1
Karitzky, J.2
Brummer, D.3
Schreiber, H.4
Haussler, J.5
Ludolph, A.C.6
Hanemann, C.O.7
-
88
-
-
12944263711
-
The Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcription
-
Steffan J.S., Kazantsev A., Spasic-Boskovic O., Greenwald M., Zhu Y.Z., Gohler H., Wanker E.E., Bates G.P., Housman D.E., and Thompson L.M. The Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcription. Proc. Natl Acad. Sci. USA 97 (2000) 6763-6768
-
(2000)
Proc. Natl Acad. Sci. USA
, vol.97
, pp. 6763-6768
-
-
Steffan, J.S.1
Kazantsev, A.2
Spasic-Boskovic, O.3
Greenwald, M.4
Zhu, Y.Z.5
Gohler, H.6
Wanker, E.E.7
Bates, G.P.8
Housman, D.E.9
Thompson, L.M.10
-
89
-
-
0035909330
-
Histone deacetylase inhibitors arrest polyglutamine-dependent neurodegeneration in Drosophila
-
Steffan J.S., Bodai L., Pallos J., Poelman M., McCampbell A., Apostol B.L., Kazantsev A., Schmidt E., Zhu Y.Z., Greenwald M., Kurokawa R., Housman D.E., Jackson G.R., Marsh J.L., and Thompson L.M. Histone deacetylase inhibitors arrest polyglutamine-dependent neurodegeneration in Drosophila. Nature 413 (2001) 739-743
-
(2001)
Nature
, vol.413
, pp. 739-743
-
-
Steffan, J.S.1
Bodai, L.2
Pallos, J.3
Poelman, M.4
McCampbell, A.5
Apostol, B.L.6
Kazantsev, A.7
Schmidt, E.8
Zhu, Y.Z.9
Greenwald, M.10
Kurokawa, R.11
Housman, D.E.12
Jackson, G.R.13
Marsh, J.L.14
Thompson, L.M.15
-
90
-
-
0032945938
-
Polyglutamine-expanded androgen receptors form aggregates that sequester heat shock proteins, proteasome components and SRC-1, and are suppressed by the HDJ-2 chaperone
-
Stenoien D.L., Cummings C.J., Adams H.P., Mancini M.G., Patel K., DeMartino G.N., Marcelli M., Weigel N.L., and Mancini M.A. Polyglutamine-expanded androgen receptors form aggregates that sequester heat shock proteins, proteasome components and SRC-1, and are suppressed by the HDJ-2 chaperone. Hum. Mol. Genet 8 (1999) 731-741
-
(1999)
Hum. Mol. Genet
, vol.8
, pp. 731-741
-
-
Stenoien, D.L.1
Cummings, C.J.2
Adams, H.P.3
Mancini, M.G.4
Patel, K.5
DeMartino, G.N.6
Marcelli, M.7
Weigel, N.L.8
Mancini, M.A.9
-
91
-
-
0037408279
-
Transcriptional abnormalities in Huntington disease
-
Sugars K.L., and Rubinsztein D.C. Transcriptional abnormalities in Huntington disease. Trends Genet 19 (2003) 233-238
-
(2003)
Trends Genet
, vol.19
, pp. 233-238
-
-
Sugars, K.L.1
Rubinsztein, D.C.2
-
92
-
-
0037194896
-
Androgen-dependent neurodegeneration by polyglutamine-expanded human androgen receptor in Drosophila
-
Takeyama K., Ito S., Yamamoto A., Tanimoto H., Furutani T., Kanuka H., Miura M., Tabata T., and Kato S. Androgen-dependent neurodegeneration by polyglutamine-expanded human androgen receptor in Drosophila. Neuron 35 (2002) 855-864
-
(2002)
Neuron
, vol.35
, pp. 855-864
-
-
Takeyama, K.1
Ito, S.2
Yamamoto, A.3
Tanimoto, H.4
Furutani, T.5
Kanuka, H.6
Miura, M.7
Tabata, T.8
Kato, S.9
-
93
-
-
0028296724
-
X-linked recessive bulbospinal neuronopathy (Kennedy's syndrome): a neurophysiological study
-
Trojaborg W., and Wulff C.H. X-linked recessive bulbospinal neuronopathy (Kennedy's syndrome): a neurophysiological study. Acta Neurol. Scand 89 (1994) 214-219
-
(1994)
Acta Neurol. Scand
, vol.89
, pp. 214-219
-
-
Trojaborg, W.1
Wulff, C.H.2
-
94
-
-
0037184933
-
Ligand promotes intranuclear inclusions in a novel cell model of spinal and bulbar muscular atrophy
-
Walcott J.L., and Merry D.E. Ligand promotes intranuclear inclusions in a novel cell model of spinal and bulbar muscular atrophy. J. Biol. Chem 277 (2002) 50855-50859
-
(2002)
J. Biol. Chem
, vol.277
, pp. 50855-50859
-
-
Walcott, J.L.1
Merry, D.E.2
-
95
-
-
0033041668
-
Assessment of upper and lower motor neurons in Kennedy's disease: implications for corticomotoneuronal PSTH studies
-
Weber M., and Eisen A. Assessment of upper and lower motor neurons in Kennedy's disease: implications for corticomotoneuronal PSTH studies. Muscle Nerve 22 (1999) 299-306
-
(1999)
Muscle Nerve
, vol.22
, pp. 299-306
-
-
Weber, M.1
Eisen, A.2
-
96
-
-
0023130132
-
X-linked bulbo-spinal neuronopathy: a family study of three patients
-
Wilde J., Moss T., and Thrush D. X-linked bulbo-spinal neuronopathy: a family study of three patients. J. Neurol. Neurosurg. Psychiatry 50 (1987) 279-284
-
(1987)
J. Neurol. Neurosurg. Psychiatry
, vol.50
, pp. 279-284
-
-
Wilde, J.1
Moss, T.2
Thrush, D.3
-
97
-
-
0022462204
-
Androgen receptor levels in cranial nerve nuclei and tongue muscles in rats
-
Yu W.-H.A., and McGinnis M.Y. Androgen receptor levels in cranial nerve nuclei and tongue muscles in rats. J. Neurosci 6 (1986) 1302-1307
-
(1986)
J. Neurosci
, vol.6
, pp. 1302-1307
-
-
Yu, W.-H.A.1
McGinnis, M.Y.2
-
98
-
-
0019349967
-
Effect of testosterone and 5α-dihydrotestosterone on regeneration of the hypoglossal nerve in rats
-
Yu W.-H.A., and Srinivasan R. Effect of testosterone and 5α-dihydrotestosterone on regeneration of the hypoglossal nerve in rats. Exp. Neurol 71 (1981) 431-435
-
(1981)
Exp. Neurol
, vol.71
, pp. 431-435
-
-
Yu, W.-H.A.1
Srinivasan, R.2
-
99
-
-
0031012399
-
1A-voltage-dependent calcium channel
-
1A-voltage-dependent calcium channel. Nat. Genet 15 (1997) 62-69
-
(1997)
Nat. Genet
, vol.15
, pp. 62-69
-
-
Zhuchenko, O.1
Bailey, J.2
Bonnen, P.3
Ashizawa, T.4
Stockton, D.W.5
Amos, C.6
Dobyns, W.B.7
Subramony, S.H.8
Zoghbi, H.Y.9
Lee, C.C.10
-
100
-
-
0034094873
-
Glutamine repeats and neurodegeneration
-
Zoghbi H.Y., and Orr H.T. Glutamine repeats and neurodegeneration. Annu. Rev. Neurosci 23 (2000) 217-247
-
(2000)
Annu. Rev. Neurosci
, vol.23
, pp. 217-247
-
-
Zoghbi, H.Y.1
Orr, H.T.2
-
101
-
-
0041353535
-
Huntingtin interacts with REST/NRSF to modulate the transcription of NRSE-controlled neuronal genes
-
Zuccato C., Tartari M., Crotti A., Goffredo D., Valenza M., Conti L., Cataudella T., Leavitt B.R., Hayden M.R., Timmusk T., Rigamonti D., and Cattaneo E. Huntingtin interacts with REST/NRSF to modulate the transcription of NRSE-controlled neuronal genes. Nat. Genet 35 (2003) 76-83
-
(2003)
Nat. Genet
, vol.35
, pp. 76-83
-
-
Zuccato, C.1
Tartari, M.2
Crotti, A.3
Goffredo, D.4
Valenza, M.5
Conti, L.6
Cataudella, T.7
Leavitt, B.R.8
Hayden, M.R.9
Timmusk, T.10
Rigamonti, D.11
Cattaneo, E.12
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